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Corticobasal degeneration (CBD) is a neurodegenerative disease characterized by abnormal aggregation of hyperphosphorylated 4R-tau in cortical and subcortical areas of the brain. It is associated with various clinical phenotypes, such as the characteristic clinical phenotype corticobasal syndrome (CBS), which manifests with asymmetric akinetic–rigid, poorly levodopa-responsive parkinsonism, and cerebral cortical dysfunction. Other associated phenotypes are progressive supranuclear palsy (PSP) syndrome, frontotemporal dementia, Alzheimer’s disease (AD)-like dementia, and non-fluent/agrammatic variant of primary progressive aphasia. Precise use of terminology is critical for a common understanding in discussions of clinical phenotype, attempted clinical diagnosis of CBD with its many presenting phenotypes, and accurate pathologic diagnosis (which can only be made neuropathologically). Diagnosis of probable or possible CBS and the other CBD-associated syndromes is based on the presence of certain clinical features. Pathologic and neuroimaging findings and currently available biological markers are discussed. Treatment for CBD and CBS is symptomatic and supportive at present.
This introduction serves as a preface to the volume and provides an overview of the purpose of this Companion, and a paragraph about each essay in the collection.
This study tests whether prediction error underlies structural priming in a later-learnt L2 across two visual world eye-tracking priming experiments. Experiment 1 investigates priming when learners encounter verbs biased to double-object-datives (DO, “pay”) or prepositional-object-datives (PO, “send”) in the other structure in prime sentences. L1-German–L2-English learners read prime sentences crossing verb bias and structure (DO/PO). Subsequently, they heard target sentences – with unbiased verbs (“show”) – while viewing visual scenes. In line with implicit learning models, gaze data revealed priming and prediction-error effects, namely, more predictive looks consistent with PO following PO primes with DO-bias verbs. Priming in comprehension persisted into (unprimed) production, indicating that priming by prediction error leads to longer-term learning. Experiment 2 investigates the effects of target verb bias on error-based priming. Priming and prediction-error effects were reduced for targets with non-alternating verbs (“donate”) that only allow PO structures, suggesting learners’ knowledge of the L2 grammar modulates prediction-error-based priming.
Using high-order simulations, we have shed light on complex chemically reacting flow processes and identified new mechanisms of the supersonic combustion process. We have employed 11th-order accurate implicit large eddy simulation (ILES) in conjunction with a finite-rate (Arrhenius) thermochemistry model using a reduced reaction mechanism for the combustion of hydrogen and air. We compare the coarse-grained computations with available experiments from the German Aerospace Centre (DLR) and discuss the accuracy and uncertainties. A supersonic combustion chamber can be accurately modelled using high-order ILES without a specific turbulence-chemistry model. The simulations reveal that the flame intermittently propagates upstream behind the wedge-shaped flame holder, alternating between the upper and lower turbulent free shear layers at a frequency of ≃ 7,990 Hz. This can be a leading cause of unsteady pressure loadings on the interior surfaces downstream of the combustion chamber and is a crucial structural design parameter. Furthermore, the simulations reveal that high temperatures are sustained long distances downstream of the combustion onset. A barycentric map for the Reynolds stresses is employed to analyze the turbulent anisotropy. The results correlate the axisymmetric contraction and expansion of turbulence with the interaction of the reflected shock waves and the supersonic combustion hydroxyl production regions. The physics insights presented in this study could potentially lead to more efficient supersonic combustion and engineering designs.
Schubert polynomials are polynomial representatives of Schubert classes in the cohomology of the complete flag variety and have a combinatorial formulation in terms of bumpless pipe dreams. Quantum double Schubert polynomials are polynomial representatives of Schubert classes in the torus-equivariant quantum cohomology of the complete flag variety, but no analogous combinatorial formulation had been discovered. We introduce a generalization of the bumpless pipe dreams called quantum bumpless pipe dreams, giving a novel combinatorial formula for quantum double Schubert polynomials as a sum of binomial weights of quantum bumpless pipe dreams. We give a bijective proof for this formula by showing that the sum of binomial weights satisfies a defining transition equation.
The concluding chapter discusses the implications of Hong Kong’s contentious politics within the global context of democratic backsliding and spontaneous mass mobilizations. We highlight the contributions of our theoretical framework and the implications of Hong Kong’s contentious pathways for hybrid regimes and beyond.
Autoimmune movement disorders, although relatively rare, are not to be missed because of the treatment implications. There is a broad clinical spectrum, but recognition of some characteristic forms, or associated red flags or other clues, can point to the diagnosis. This chapter covers the clinical spectrum of primary neurologic or systemic autoimmune disease presenting or mainly manifesting as a movement disorder, and addresses the underlying immune-pathophysiologic aspects.
Despite the promise of a well-ordered trading system and expansion of trade liberalization in the 1980s and 1990s, why is trade governance deadlocked? This puzzle is addressed by elaborating the meaning of “crisis of success” and what such a crisis looks like at the global trading order. I argue that the global trading system is revealing a “crisis of success” in that its current failure to ensure functional trade liberalization change is a result of the initial accomplishment in successfully undergoing a power shift in global trade governance especially at the WTO, marked by greater inclusion of new emerging powers. The success of the initial first phase of trade governance (1990s–2008/2012), especially in aiding an institutional power shift at the global level, created conditions for new problems, leading to a fragmented turn toward preferential trade agreements as well as trade-mediated conflictual strategies rather than sustainable peaceful change. The chapter’s dynamic historical argument delineates how early successes in global trade policies and governance led to maximalist peaceful change, followed by crisis leading to greater use of tariffs and ensuing US–China economic and trade competition. The mechanisms that underwrote the paradox of crisis despite institutional strength are elaborated.
Among all the narratives collected in the Bible, none tells a story quite like the Acts of the Apostles. Readers travel along with the action over very long distances and visit a range of cultural settings in this geographically expansive tale that brings a wide spectrum of people onto the narrative stage.
Jiří Adámek, Czech Technical University in Prague,Stefan Milius, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany,Lawrence S. Moss, Indiana University, Bloomington
This chapter provides a practitioner’s point of view on diplomatic images. The author is positioned to give a unique perspective as a freelance photographer who is currently based in Singapore, which has recently become a significant city-state for major global diplomatic events. Through his first-hand experiences of covering high-profile international diplomatic events, such as the 2018 Trump-Kim Summit held in Singapore, the author takes us backstage and demonstrates how famous diplomatic images are produced to represent the affective register of the moment. In so doing, the chapter illuminates the situational context of the photographer in taking diplomatic images, offering insight into the editorial process in which diplomatic images are produced by the media.